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  • ISSN: 2373-9312
    Special Issue on Pediatric Gastroenterology Disorders
    Special Issue on Pediatric Gastroenterology Disorders
    Hillel Naon
    Interim Division Head
    Pediatric Gastroenterology, Hepatology
    Nutrition and Liver Transplant
    Children's Hospital Los Angeles, USA
    Research Article
    Tara Harwood1, Shrinidi Mani1*, Lisa Feinberg1, Sarah Worley2 and Naim Alkhouri1
    Objectives: Incomplete fructose absorption can result in gastrointestinal distress symptoms in children, which may impact their quality of life; however, this has not been formally studied. This study evaluates whether dietary fructose restriction improves quality of life and gastrointestinal symptoms in children with breath test confirmed fructose intolerance, as measured by the Pediatric Quality of Life Inventory (PedsQL) and PedsQL Gastrointestinal Symptom Scale (GSS).
    Methods: Thirty eight subjects, ages ranging from 8 to 18 years old with a positive hydrogen breath test consistent with fructose intolerance were included. Subjects drank 2 grams of fructose sugar per every kilogram of body weight, with a maximum of 50 grams fructose sugar. Hydrogen was collected every 30 minutes over two hours. Hydrogen production of >20 ppm indicated a positive test. Participants with a positive breath test completed a PedsQL and a GSS assessment worksheet prior to leaving the office on the day of diagnosis. A dietitian then instructed subjects to follow a two-week fructose restricted diet. Subjects were then called exactly 2 weeks after diagnosis, and a post-intervention PedsQL/GSS was administered.
    Results: The median age of subjects studied was 12.2 (10.4, 15.1) years. GSS and PedsQL scores showed statistically significant improvement from a mean of 47.2 (± 14) to 72.8 (± 15.5) and 75.0 (13.3) to 88.3 (± 8.9) respectively after the two-week dietary intervention (p value < 0.001 for both). Subjects showed significantly improvement on their total PedsQL score and in 4 out of the 5 PedsQL subscales, namely physical, emotional, school, and psychosocial function (p value < 0.001 for all).
    Conclusion: Fructose restricted diet can significantly improve quality of life in fructose intolerant children.
    Alexander Koral1, Richard Quan2, James P. Marcin3, Michael Haight4 and Antonio J Quiros5*
    Background: In adults, it has been demonstrated that nighttime gastroesophageal reflux (GER) occurs primarily in the first half of sleep. It is unknown if the same reflux pattern occurs in children with GER.
    Objective: To characterize the association between age and GER symptoms with the frequency and severity of nighttime GER in children.
    Study Design: Esophageal pH monitoring studies performed in 135 symptomatic children were analyzed retrospectively. Children were divided into 3 age groups: Children <1 year of age, children between 1 and 3 years of age, and children greater than 3 years of age. Nighttime was defined as the period from 6 pm to 6 am, and sub-divided into early and late nighttime (6 pm to 12 midnight, 12 midnight to 6 am, respectively). The total number of acid reflux episodes, reflux index (RI), and esophageal acid clearance time (AC) was monitored and compared between different periods throughout the monitoring period. We also examined the association between clinical symptoms of acid reflux and objective measures of GER severity.
    Results: Children <3 years of age had a higher frequency of acid reflux during the daytime (63.5 versus 33.0, p<0.01), nighttime (46.0 versus 19.0, p<0.01), early nighttime (29.0 versus 13.0, p<0.01) and late nighttime (14.5 versus 2.0, p<0.01) compared to children =3 years of age. Children less than 1 year of age had similar acid reflux episodes during the early and late-night, whereas children 1-3 and =3 years of age had more frequent early nighttime acid reflux than late nighttime acid reflux. We found no consistent association between clinical symptoms and severity of acid reflux, other than a higher frequency of acid reflux episodes among children with complaints of vomiting.
    Conclusions: There are a significantly more episodes of nighttime GER among children <3 years of age compared to children =3 years of age, and more frequent acid reflux during the early nighttime compared to the late nighttime only among children =3 years of age. This may be a result of more frequent nighttime feeding among younger children and infants. We also found that the RI and AC were not significantly different between the early and late nighttime periods among all patient age groups, which contrast with data from adult studies.
    Brynie Slome Collins, Tanaz Farzan Danialifar*, Neena Kapoor and Hillel Naon
    Objectives: Graft versus host disease (GVHD) is the most common cause of diarrhea in allogeneic hematopoietic progenitor cell transplant (HPCT) recipients. Diarrhea may be caused by infection or epithelial cell damage secondary to chemotherapy or radiation, which in turn can decrease brush border enzyme activity. Disaccharidase levels and the potential for lactose malabsorption as a contributing factor for diarrhea have not been studied in patients with GVHD. The aim of this study is to evaluate the prevalence of disaccharidase deficiency in pediatric HPCT patients with GVHD and its potential role in causing diarrhea.
    Methods: A retrospective review of 21 HPCT patients with protracted diarrhea referred for gastrointestinal endoscopy and biopsy to rule out GVHD. Biopsies were evaluated for infection, GVHD, or other abnormalities. Duodenal samples were frozen and sent for disaccharidase analysis.
    Results: One patient was excluded from the study because disaccharidase analysis was not sent. Grade 1 GVHD was found in 11 patients (55%), grade 2 in 1 patient (.05%), and no GVHD in the remaining 8 patients (40%). Ten patients with GVHD (83%) and 6 without (75%) had an isolated lactase deficiency (p =0.27). Six patients had virus present on intestinal biopsies and 1 patient had C. Difficile infection.
    Conclusions: There is not a greater prevalence of either isolated lactase or global disaccharidase deficiency in pediatric HPCT patients with Grade 1 or 2 GVHD. However, regardless of presence of GVHD, the majorities of HPCT patients with protracted diarrhea have isolated lactase deficiency and may benefit from a lactose-free diet.
    Benjamin R Doolittle*
    Background: Neighborhood safety and certain behaviors - namely doing homework and reading - have not been widely correlated with obesity.
    Methods: Convenience sample of 124 patients between 7-18 years old from an urban, impoverished community
    Results: Forty seven patients (38%) met criteria for obesity. The children spend much of their time watching television (86 (69%) spend 1-7 hours per week), playing video games (84 (68%) spend 1-7 hours per week), and homework (80 (65%) spend 1-7 hours per week). Most children are not involved in extracurricular activities: Sixty eight (55%) do not play organized sports, 109 (89%) do not participate in social clubs. Only lack of reading (LR=7.2, p=0.01) and lack of doing homework (LR= 3.5, p=0.05) were associated with increased obesity. Perception of neighborhood safety was not associated with increased obesity.
    Conclusions: In this population where prevalence of obesity was high, lack of reading and doing homework was correlated with increased prevalence of obesity.
    Review Article
    Vrinda Bhardwaj*, Rula Harb and Hillel Naon
    Eosinophilic esophagitis (EoE) is a chronic, atopic inflammatory disorder of the esophagus. With the escalating prevalence in children and young adults, revised guidelines have been established. Its pathogenesis has been associated with T helper Type 2 (Th2) inflammatory response. Diagnosis is based on a comprehensive evaluation including patient's clinical manifestations and characteristic histologic findings on esophageal mucosal biopsies. The management of EoE involves dietary approaches and pharmacologic agents. This article provides a practical overview of recent literature pertaining to the epidemiology, pathophysiology, diagnosis, treatment, and prognosis of EoE.
    Michelle Pietzak1* and Hillel Naon2
    Wheat has been a staple in the human diet for over 10,000 years. However, in contrast to ruminant animals, humans did not evolve to digest wheat gluten completely before it leaves our one-chambered stomach. Response to the dietary proteins in wheat can result in a variety of symptoms and immunologic responses. In celiac disease, genetically predisposed patients react to the gliadin fraction of wheat gluten, and this result in a variety of intestinal and extra-intestinal manifestations. In contrast, wheat allergy is typically an IgE mediated response, resulting in gastrointestinal, skin and/or respiratory symptomatology. Non-celiac gluten sensitivity is a relatively new entity, and has an evolving definition. It is thought to occur when patients who have neither celiac disease nor wheat allergy exhibit symptom improvement upon dietary gluten withdrawal. Conditions in this category include dermatitis herpetiformis, irritable bowel syndrome, autism and gluten-sensitive ataxia. All of these entities respond to a gluten free diet, but is it important for patients and health care practitioners to understand the distinctions. The potential health implications of differentiating among an "autoimmune" disorder, "allergy" and "sensitivity" are broad. Significant morbidity can result if the specific condition is not identified and treated correctly.
    Sonia Michail*, Ann Marie Botros and RulaHarb
    Probiotics are live microorganisms that have recently gained popularity for being beneficial to human health and digestion. There are several forms of probiotics, perhaps the more widely used form is the lactic acid producer. Studies with the strongest clinical evidence have shown beneficial effects of probiotics for acute infectious diarrhea in children, antibiotic-associated diarrhea, among several others. Some studies have shown promising prophylactic effects for necrotizing enterocolitis, the common cold, irritable bowel syndrome, cancer, mucosal immunity, allergies, and urinary tract infections. While probiotics are generally known to be safe, there are occasional reports of adverse effects. Chief among the safety concerns is the ability of these organisms to invade the blood stream causing bacteremia or fungemia. Another concern relates to modifying the gut immune system, the largest immune organ, at a young age if probiotics are given to infants. With regards to lactic acid-producing probiotics, there is concern that accumulation of lactic acid in the body and the development of lactic acidosis can cause clinically significant problems. This article will provide an update on the safety and efficacy of probiotics and summarize the current literature about probiotics, including the issue of quality control.
    Sonia Michail* and Alexander Klonoff
    Fecal micro biota transplant (FMT) has been introduced several decades ago in an attempt to restore the gut microbial balance. FMT appears to be the most efficient method to effectively change and sustain the gut microbial composition. To this date there have been a great number of reports of success in eliminating recurrent Clostridium difficult infections and restoring the gut microbial profile to resemble that of the healthy donor. The new gut microbiome appears to be stable in the recipients for at least 24 weeks. The efficacy of this "relatively novel" intervention in this difficult to treat population is 90%. This is superior to any other therapeutic modality, yet effective when all other therapeutic avenues have failed. While over 300 cases have been described in the literature, it has been difficult for the pediatric scientific community to embrace this therapy as there are only sporadic reports in children. FMT has also been used to treat inflammatory bowel disease, especially ulcerative colitis. There have been a number of successful case reports in ulcerative colitis, to suggest control of disease activity and a cure in some cases. However, there has not been uniform success reported for the use of FMT, especially in severe cases recalcitrant to medical therapy. Therefore, there is a strong need to determine the safety and efficacy of FMT in future pediatric randomized controlled studies, especially in inflammatory bowel disease. This review describes the rationale for fecal transplant and provides an update on the current published studies.
    A-Kader HH1*, Feerick J1 and Rodriguez-Davalos M2
    Two centuries after Professor John Burns, of the Department of Surgery, University of Glasgow referred to biliary atresia in his text book the etiology of the obliterative process remains a mystery. We still lack a complete understanding of the pathogenesis of the disorder, which hurdled our efforts to develop preventive and therapeutic measures. Several genetic, immune, viral, toxic and vascular etiologies have been proposed without conclusive evidence. Early recognition and prompt intervention are imperative in order to prevent rapid progressive damage to the liver. Although liver transplantation has been proposed as a primary therapy, the Kasai procedure (hepatoportoenterostomy remains the most reasonable initial approach. Biliary atresia is the most common indication for liver transplantation in the pediatric age group accounting for at least 50% of all transplants.
    Nanda Kerkar1* and Hillel Naon2
    Autoimmune liver disease in children encompasses autoimmune hepatitis (AIH), AIH overlapping with sclerosing cholangitis, drug induced AIH, AIH in syndromes, systemic disease and AIH after liver transplantation (LT). AIH is an uncommon progressive disease of the liver of unknown etiology affecting all ethnic groups and with female predilection. Characteristic clinical findings include elevated transaminases, elevated total Immunoglobulin G, positive autoantibodies and interface hepatitis on liver histology, in the absence of other known causes of liver disease. A scoring system is available to enable diagnosis given there is no single pathognomonic feature for AIH. AIH is designated as type 1 when there is positivity for antinuclear antibody +/- smooth muscle antibody and type 2 when there is positivity for liver kidney microsomal antibody demonstrated. Clinical presentation can range from asymptomatic to acute hepatitis like picture, jaundice, acute liver failure, and end-stage liver disease. AIH is characteristically treated with immunosuppression. Patients who have overlap with sclerosing cholangitis respond biochemically to ursodeoxycholic acid (UDCA), but effect on long-term outcome in children is unclear. LT is indicated in acute liver failure with encephalopathy or end-stage liver disease. AIH may recur after LT and also may occur denovo in a proportion of children transplanted for conditions other than AIH.
    Case Report
    Alexander Wu1, Lauren Alessi2, Allyson Wyatt3 and Arvind Srinath3*
    Introduction: Foreign body ingestion is a common problem in the pediatric population. Specifically, magnet ingestion, which has unique and potentially life-threatening consequences, has increased significantly in the last decade.
    Case Presentation: A 13-year-old female with severe autism presented with vague abdominal discomfort and bloating. Abdominal films confirmed multiple foreign bodies in the stomach and small bowel.The gastric foreign bodies were found to be magnets and successfully removed endoscopically. The magnets in the small bowel were found to have perforated into the cecum on colonoscopy and therefore, surgery was deemed necessary.
    Conclusion: While foreign body ingestion is most commonly seen in young children, other pediatric populations, such as children with autism, are also at risk. The symptoms of magnet ingestion can be vague, but the complications are life-threatening. Therefore, familiarity with the common symptoms and early recognition are crucial for the management of these cases.
    Brigitte Moreau1*, Van-Hung Nguyen2, Sharon Abish3 and Terry Sigman4
    Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the gastrointestinal tract. They are rarely seen in children, and the majority of cases occur in the stomach. We report the case of a 17 years old male with a duodenal GIST. He presented with the sole symptom of acute onset fatigue and shortness of breath and on admission was found to have a severe microcytic anemia. An upper endoscopy was performed and a polypoid mass with an adherent clot was identified in the duodenum. No obvious stalk was seen and a biopsy of the mass was taken. Histological findings of the biopsy revealed a spindle cell GIST positive for C-KIT (CD117), a type III tyrosine kinase receptor. Ten days after his admission, the tumor was resected. The mitotic rate was 9/ 50 HPF with negative margins which conferred an intermediate risk according to the NIH consensus. C-KIT positivity was confirmed. Mutation analysis was performed and was positive for mutation in KIT gene exon 11. No mutation was detected in the PDGFRA exons. Typically, gastric GIST is described on endoscopy as a submucosal mass with smooth margins and a normal overlying mucosa. Endoscopic biopsies are often reported as nondiagnostic. In this case, the mass was polypoid and friable in appearance and was suspicious for a polyposis syndrome. This case substantiates the importance of including GIST in the differential diagnosis of a duodenal polypoid mass on endoscopy. Furthermore, it demonstrates the usefulness of endoscopic biopsy in confirming the diagnosis, which can help in the surgical management of these patients.
    Harry A. Cynamon1*, Hillel Naon1 and Deepti Dhall2
    Collagenous gastritis is a rarely diagnosed disorder in pediatrics. The pediatric onset type typically presents with abdominal pain and anemia and the adult onset type with diarrhea. We present 2 new cases of collagenous gastritis. The first case presented with abdominal pain but no anemia. The second case presented with diarrhea as seen in the adult onset form and found to have celiac disease. This is the second report in a pediatric patient with collagenous gastritis and proven celiac disease.
    Jason Williams1, Paul Thacker2 and Antonio J Quiros3*
    A type III choledochal cyst (choledochocele) is a rare lesion affecting the distal common bile duct and pancreato-biliary junction, which is consistently described in the literature as a cystic lesion involving the duodenal wall at the ampulla and involving these structures. The exact etiology is unknown with some hypothesizing that this is a congenital anomaly while others supporting that a choledochocele is acquired. This case presents a 7 year-old male child with recurrent pancreatitis and a choledochocele having a varied appearance over time on magnetic resonance cholangiopancreatography (MRCP). Patient was amply imaged, a choledochocele due to an ampullary obstruction was suspected and he underwent a therapeutic endoscopic intervention (ERCP) with resulting complete resolution of his symptoms. This case supports the hypothesis that these lesions are actually acquired and related to obstruction within the distal ampulla. Lastly, this case highlights the diagnostic utility of MRCP in the pediatric patient and ERCP as a therapeutic option for these lesions.
    Tania F Mitsinikos1*, Hillel Naon1, Nick Shillingford2, Chuan-Hao Lin1 and Rula Harb1
    Background: Cytomegalovirus (CMV) is a ubiquitous virus and has significant implications in the immune suppressed. Several cases of CMV colitis have been reported in neonates, acquiring the virus in a variety of ways, through vertical transmission, breast milk, or blood transfusion.
    Case Presentation: Here we report a full-term infant who developed severe cytomegalovirus colitis post-operatively after imperforate anus repair.
    Conclusion: In many cases, identification of transmission of CMV is difficult and often times unidentified, even in the case presented here. Discussion of congenital CMV infection is discussed and considered unlikely.
    Brigitte Moreau1*, Dorothee Dal Soglio2, Colette Deslandres3 and Ernest G Seidman1
    Inflammatory bowel disease (IBD) is a known risk factor for colorectal cancer (CRC). The degree of risk is related to the duration and extent of colitis, as well as the severity of inflammation over time. We present a 17 years old female diagnosed with carcinoma in situ associated with ulcerative colitis (UC) at first colonoscopy for suspected IBD. She presented with non-bloody diarrhea of 3 years' duration. A clinical diagnosis of irritable bowel syndrome (IBS) was suggested elsewhere and her symptoms improved with loperamide. Hematochezia triggered an investigation for IBD. The colonoscopy revealed pancolitis and no tumor was seen. Histology confirmed characteristic signs of chronic colitis with focal ulcerations, crypt branching and abscesses and dense acute and chronic inflammatory infiltrates. Multiple biopsies showed signs of definite dysplasia, as well as the presence of P53 and k167 expression. Two independent pathologists confirmed high-grade dysplasia with carcinoma in situ in the descending colon. A proctocolectomy with ileo-anal J pouch anastomosis was performed. The pathology specimen confirmed a diagnosis of UC without skip lesions and a carcinoma in situ in the descending colon.
    Rare cases of CRC at or shortly after diagnosis of IBD exist. Our case is exceptional in that CRC was found at the time of initial colonoscopy for suspected IBD. This emphasizes the fact that CRC can be present early after the onset of symptoms and may be delayed or missed when conducting surveillance strictly according to formal guidelines.
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